What causes Necrobiotic Xanthogranuloma?

Necrobiotic xanthogranuloma is a chronic, progressive, multiorgan disease of unknown cause. Given the rarity of NXG, no controlled therapeutic trials have been conducted to date; thus, prognostic factors have not been well established and no first-line therapy is recommended.

What is the cause of necrobiosis lipoidica?

The cause of necrobiosis lipoidica diabeticorum (NLD) is unknown. It is thought to be linked to blood vessel inflammation related to autoimmune factors. This damages proteins in the skin (collagen). People with type 1 diabetes are more likely to get NLD than those with type 2 diabetes.

What are the symptoms of necrobiosis lipoidica?

Signs and symptoms of necrobiosis lipoidica Patients usually present with shiny, asymptomatic patches that slowly enlarge over months to years. The patches are initially red-brown and progress to yellow, depressed, atrophic plaques. Ulcerations can occur, typically after trauma and occasionally with associated pain.

What is Necrobiotic granuloma?

A collagenolytic or necrobiotic non-infectious granuloma is one in which a granulomatous infiltrate develops around a central area of altered collagen and elastic fibers. The altered fibers lose their distinct boundaries and exhibit new staining patterns, becoming either more basophilic or eosinophilic.

What causes diabetic dermopathy?

Diabetic dermopathy, also known as shin spots or pigmented pretibial patches, is a skin condition usually found on the lower legs of people with diabetes. It is thought to result from changes in the small blood vessels that supply the skin and from minor leakage of blood products from these vessels into the skin.

How is NLD treated?

First-line therapy for NLD includes nonsteroidal inflammatory agents, cryotherapy, and potent topical glucocorticoid agents for early lesions and intralesional corticosteroids injected into the active borders of established lesions.

How do you get rid of necrobiosis lipoidica?

According to W.R. Heymann, tretinoin has been used to diminish the atrophy associated with necrobiosis lipoidica (personal communication). Durupt et al reported successful treatment of necrobiosis lipoidica with chloroquine and hydroxychloroquine. Improvement was seen within 3- 6 months of treatment.

What is the difference between necrobiosis and necrobacillosis?

Necrobiosis in humans (generally due to biovar B) is a throat infection followed by systemic spread and metastatic abscesses. Necrobacillosis infections in animals (generally due to biovar A) are usually polymicrobial and include coagulative necrosis and abscesses and can cause mortality.

What is the pathophysiology of necrotic necrosis glaucoma (NXG)?

Histopathologically, NXG is characterized by a granulomatous inflammation in the dermis extending into the subcutaneous fat. There are large zones of necrobiosis (altered collagen with sheets of histiocytes and inflammatory cells) surrounded by granulomas composed of macrophages with some foreign body-type and Touton giant cells (Figure 2).

What are the possible complications of nerve necrosis (NXG)?

In the skin, NXG can ulcerate and scar, either spontaneously or as a result of iatrogenic intervention, leading to disfigurement. NXG can have multiple systemic associations, leading to extracutaneous complications: Ophthalmologic complications: Ophthalmologic symptoms include burning, itching, or painful eyes.

How is necrobiosis lipoidica differentiated from sarcoidosis?

The most common entities to differentiate against include: necrobiosis lipoidica (pretibial location), xanthelasma (periorbital but less infiltrating plaques), sarcoidosis (less typically yellow/orange in color), and granuloma annulare (flesh and pink/red in color with more annular appearance, rarely periorbital).